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Reconhecido pela Organização Mundial de Saúde em 2016, o linfoma anaplásico de grandes células associado ao implante mamário (BIA-ALCL) é um subtipo incomum de linfoma não Hodgkin de células T, que se desenvolve após a inserção de próteses mamárias. A doença é uma afecção rara que afeta cerca de uma a cada 30.000 pessoas com implante mamário texturizado. As principais manifestações clínicas são o seroma tardio, assimetria mamária, massa e contratura capsular, com frequência mais elevada do primeiro. O explante da prótese com capsulectomia total pode ser suficiente para tratar o ALCL, com ressecções estendidas a locais adjacentes, quando necessário. Entretanto, em alguns casos, é realizada a radioterapia e/ou quimioterapia adjuvante. Conclui-se que, para um diagnóstico precoce e um tratamento efetivo, mulheres com seroma de aparecimento súbito e tardio deverão realizar exames complementares para a exclusão dessa afecção, mesmo com tempo inferior à média de desenvolvimento, que é de cerca de 10,6 anos.
Recognized by the World Health Organization in 2016, breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is an uncommon subtype of T-cell non-Hodgkin lymphoma that develops after the insertion of breast implants. The disease is a rare condition that affects approximately one in every 30,000 people with textured breast implants. The main clinical manifestations are late seroma, breast asymmetry, mass, and capsular contracture, with a higher frequency of the former. Explantation of the prosthesis with total capsulectomy may be sufficient to treat ALCL, with resections extended to adjacent sites when necessary. However, in some cases, adjuvant radiotherapy and/or chemotherapy is performed. It is concluded that, for an early diagnosis and effective treatment, women with sudden and late-onset seroma should undergo additional tests to exclude this condition, even with a shorter development time than the average, which is around 10.6 years.
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El cáncer de mama sigue siendo una de las principales prioridades en salud global y salud pública y permanece como la neoplasia maligna más frecuente y mortal en mujeres en el mundo. El linfoma anaplásico de células grandes asociado a implante mamario (LACG-AIM) consiste en un linfoma no-Hodgkin de tipo raro, del cual se desconoce mucho sobre su patogenia y fisiopatología, pero que se ve cada vez con mayor frecuencia, debido al aumento de procedimientos estéticos. A la fecha, existen limitaciones en cuanto al conocimiento sobre el comportamiento clínico y se manifiesta de muchas formas, con un tiempo de evolución variable, y desenlaces quirúrgicos inciertos a mediano y largo plazo. Con base en lo anterior, el objetivo de esta revisión consiste en resumir evidencia sobre las consideraciones clínicas y desenlaces quirúrgicos del cáncer asociado a implante mamario, que faciliten la identificación y abordaje de esta condición. Se realizó una búsqueda bibliográfica en los motores de búsqueda y bases de datos PubMed, ScienceDirect, Embase, EBSCO y MEDLINE. Dentro de las consideraciones clínicas y quirúrgicas, se debe tener en cuenta el tipo de implante utilizado (texturizado), el tiempo del antecedente del implante, la severidad de las manifestaciones y la estadificación, para poder determinar la oportunidad de intervención quirúrgica y terapia neoadyuvante e intentar garantizar la supervivencia y evitar recurrencia. Aquellos pacientes sometidos a capsulectomía completa acompañado de radioterapia tienen mejores desenlaces.
Breast cancer continues to be one of the main priorities in global health and public health, and remains the most frequent and deadly malignant neoplasm in women worldwide. Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a rare type of Non-Hodgkin's lymphoma, whose pathogenesis and pathophysiology are not well known, but which is seen with increasing frequency due to the increase in cosmetic procedures. To date, there are limitations in terms of knowledge about the clinical behavior of the disease, which can manifest itself in many forms, with a variable evolution time and uncertain surgical outcomes in the medium- and long-term. Based on the above, the aim of this review is to summarize evidence on the clinical considerations and surgical outcomes of breast implant-associated cancer to facilitate the identification and management of this condition. A bibliographic search was performed in the search engines and databases pubmed, sciencedirect, embase, ebsco and medline. Within the clinical and surgical considerations, the type of implant used (textured), the time of the implant history, the severity of the manifestations, and the staging, must be taken into account in order to determine the opportunity for surgical intervention and neoadjuvant therapy, and to try to guarantee survival and avoid recurrence. Patients who undergo complete capsulectomy with radiotherapy have better outcomes.
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Objective:To investigate clinical and laboratory characteristics of secondary hemophagocytic lymphohistiocytosis (sHLH) associated with secondary cutaneous T-cell lymphoma (CTCL) .Methods:CTCL patients with clinically suspected sHLH were collected from Department of Hematology, Wuhan No.1 Hospital from January 2016 to October 2021, and were evaluated according to the HLH-2004 diagnostic criteria and HScore.Results:Seven CTCL patients were confirmedly diagnosed with sHLH, including 2 with primary cutaneous γδT-cell lymphoma (PC-GDTCL) , 3 with cutaneous extranodal natural killer/T-cell lymphoma (C-ENKTCL) , and 2 with primary cutaneous anaplastic large cell lymphoma (PC-ALCL) . All the 7 patients received chemotherapy, but 6 died finally, and the median overall survival duration was 26.5 days (range: 14 - 60 days) after the confirmed diagnosis of CTCL complicated by sHLH. HLH-related gene mutations, which were located in the PRF1 and LYST genes, were identified in 2 patients; lymphoma-related gene mutations were identified in the KRAS and KMT2D genes in 1 PC-GDTCL patient,and in the JAK3 and SAMHD1 genes in another PC-GDTCL patient.Conclusions:CTCL complicated by sHLH usually progresses rapidly, so early diagnosis and treatment are needed. Bone marrow biopsy and mutation screening of lymphoma- and HLH-related genes at initial diagnosis and during disease progression may facilitate early diagnosis.
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Abstract Intratumoral similarities and differences between large-cell neuroendocrine carcinomas (LCNECs) and small-cell lung carcinomas (SCLCs) are determined partially by the Notch signaling pathway, which controls the switch from neuroendocrine to slight/non-neuroendocrine cell fate. LCNECs are divided into two subgroups according to genomic alterations: type I LCNECs exhibit a neuroendocrine profile characterized by achaete‐scute homolog 1 (ASCL1)high/delta-like protein 3 (DLL3)high/NOTCHlow and type II LCNECs show the pattern ASCL1low/DLL3low/NOTCHhigh. Here, we used immunohistochemistry, transmission electron microscopy, and digital analysis to examine the role of the Notch ligand DLL3 as an immunomarker of the neuroendocrine state and ASCL1 as a regulator of cell-cell interactions in SCLCs and LCNECs. High DLL3 and ASCL1 expression was associated with atypical submicroscopic characteristics involving nuclear size, chromatin arrangement, Golgi apparatus, and endoplasmic reticulum, and was characteristic of type I LCNECs with similarity to SCLCs, whereas low DLL3 and ASCL1 expression was found in both SCLCs and type II LCNECs. In patients diagnosed at an early stage who did not have metastasis and who underwent chemotherapy, DLL3high and ASCL1high SCLCs and type I LCNECs were associated with a better prognosis and a lower risk of death. The present findings suggested that DLL3/ASCL1 are potential therapeutic targets and prognostic indicators in patients with SCLCs or LCNECs.
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ABSTRACT Laryngeal cancer ranks third among the most common head and neck neoplasms. The most common histological subtype is squamous cell carcinoma, and neuroendocrine tumors are rare. An even rarer entity is a composite tumor with both these histologies. This case reports a metastatic combined carcinoma of squamous cells and large neuroendocrine cells, presenting favorable response to treatment with a total laryngectomy followed by adjuvant therapy including chemo-, radio-, and immunotherapy.
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Introdução: Em 1963 Cronin e Gerow introduziram o uso do implante de silicone e seu uso aumentou exponencialmente. Contudo, complicações relacionadas aos implantes surgiram ao longo do tempo. O conjunto de situações adversas ao uso dos implantes de silicone, alimentado pelo crescimento das mídias sociais, culminou em um aumento da retirada definitiva do implante. Muitos casos de explante têm o pedículo inferior comprometido pela lesão dos vasos perfurantes e a técnica dos retalhos cruzados é uma alternativa para a reconstrução das mamas explantadas. Métodos: Foram realizados explantes de silicone com reconstrução imediata da mama sem o uso de um novo implante, motivados por indicação médica ou por desejo próprio do paciente. A técnica dos retalhos cruzados foi utilizada em todos os casos. Ela se vale do cruzamento de retalhos parenquimatosos de pedículo superior, um medial e outro lateral, conforme descrito por Sperli. Resultados: Foram operados 10 casos de 2004 a 2021. O tempo de uso das próteses variou de 3 a 19 anos e a principal motivação para o explante foi contratura capsular. Nenhum caso de necrose foi observado. Conclusões: A técnica dos retalhos cruzados é uma alternativa útil e segura para as cirurgias de reconstrução da mama após explante definitivo.
Introduction: In 1963 Cronin and Gerow introduced the use of the silicone implant and its use increased exponentially. However, complications related to implants emerged over time. The set of adverse situations to the use of silicone implants fueled by the growth of social media culminated in an increase in the permanent removal of the implant. Many cases of explants have the inferior pedicle compromised by injury to the perforating vessels, and the crossed flap technique is an alternative for the reconstruction of explanted breasts. Methods: Silicone explants were performed with immediate breast reconstruction without the use of a new implant, motivated by medical indication or the patients own desire. The crossed flap technique was used in all cases. It uses the crossing of parenchymal patches of the superior pedicle, one medial and one lateral, as described by Sperli. Results: 10 cases were operated from 2004 to 2021. The time of use of the prostheses ranged from 3 to 19 years and the main motivation for the explant was capsular contracture. No cases of necrosis were observed. Conclusions: The crossed flap technique is a useful and safe alternative for breast reconstruction surgeries after definitive explantation.
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Background and Aim: Anaplastic lymphoma kinase (ALK)-positive large B-cell lymphomas (ALK+-LBCLs) are aggressive CD20-negative lymphomas, accounting for <1% of diffuse LBCLs. Being rare and with peculiar immunophenotypic characteristics, these can be easily misdiagnosed. We present 11 cases of ALK+-LBCLs diagnosed over a period of 11 years at a tertiary care hospital in South India to analyze the clinical, morphological, and immunophenotypic profile of these tumors. Subjects and Methods: ALK+-LBCL cases diagnosed from September 2009 to August 2020 were included. Clinical details were obtained from stored electronic records and summarized. Available hematoxylin and eosin (H and E) stained slides and immunohistochemistry slides were reviewed and observations tabulated. Results: Eleven patients (nine males and two females) were diagnosed with ALK+-LBCLs in the study period with seven presenting primarily with extranodal disease manifestations. Tumors in the lymph nodes showed diffuse architecture effacement and variable sinusoidal invasion. All tumors showed immunoblastic and plasmablastic-type large lymphoid cells with scattered anaplastic/multinucleate large cells, including rare Reed–Sternberg-like cells. Cytoplasmic granular ALK-1 staining, CD20 negativity, and immunohistochemical features of plasmablastic differentiation were noted in all. Of eight patients treated, only one achieved remission with multi-agent chemotherapy but relapsed after 6 months. Two patients died of disease and five others had progressive/persistent disease and were lost to follow-up. Conclusion: Although rare, these tumors should always be in the differential diagnoses of tumors with plasmablastic and immunoblastic morphology, especially in extranodal sites to avoid diagnostic delay/misdiagnosis.
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Neuroendocrine carcinoma of the cervix is a rare aggressive tumor with a poor prognosis. Neuroendocrine carcinomas account for 1–1.5% of cervical cancer and around 12.5% were of large cell types. We report the case of a 55-year-old woman who came with complaints of pain abdomen and was reported as large cell neuroendocrine carcinoma of the cervix on biopsy. Immunohistochemistry was done which confirmed the diagnosis.
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Objective:To determine lysophosphatidic acid receptor 6 (LPAR6) expression in patients with mycosis fungoides (MF) , a variant of cutaneous T-cell lymphoma (CTCL) , and to investigate its role and mechanism of action in the development and prognosis of CTCL.Methods:A total of 110 patients with confirmed MF were collected from Department of Dermatology, Peking University First Hospital from 2011 to 2020, including 24 with large-cell transformation (LCT) and 25 with non-large cell transformation (NLCT) in the discovery cohort, and 24 with LCT and 37 with NLCT in the validation cohort. RNA sequencing and RT-PCR were conducted to determine the LPAR6 expression in patients in the discovery cohort and validation cohort respectively. LPAR6 expression was compared between patients with LCT and those with NLCT, and its effect on the prognosis of patients was evaluated. Two LPAR6-overexpressing CTCL cell lines MyLa and Sz4 were constructed to evaluate the effect of LPAR6 overexpression on proliferative activity of MyLa and Sz4 cells, with the cells normally expressing LPAR6 as the control group; after the treatment with LPAR6-related ligand lysophosphatidic acid (LPA) , 2S-OMPT, adenosine triphosphate (ATP) or adenosine (ADO) , the effects of LPAR6 activation on the proliferative activity and apoptosis of LPAR6-overexpressing MyLa and Sz4 cells were evaluated by the MTS method and flow cytometry respectively. Log-rank test was used for prognostic analysis, and t test or Mann-Whitney U test was used for comparisons between two groups. Results:As RNA sequencing showed, LPAR6 was one of the significantly underexpressed genes in the LCT group in the discovery cohort; in the validation cohort, LPAR6 expression (median[ Q1, Q3]) was significantly lower in the LCT group (204.90[81.90, 512.70]) than in the NLCT group (809.40[417.50, 1 829.20], U= 242.00, P= 0.002) ; in the two cohorts, the underexpression of LPAR6 was significantly associated with increased risk of poor prognosis (both P < 0.01) . Cell proliferation assay showed no significant difference in the proliferative activity of MyLa or Sz4 cells between the LPAR6 overexpression group and control group at 0, 24, 48 and 72 hours during the experiment (all P > 0.05) ; 48 hours after activation of LPAR6 by LPA, 2S-OMPT, ATP and ADO in MyLa cells, the LPAR6 overexpression group showed significantly decreased cellular proliferative activity (1.38 ± 0.01, 1.04 ± 0.01, 1.09 ± 0.03, 1.23 ± 0.01, respectively) compared the control group (1.73 ± 0.04, 1.23 ± 0.01, 1.24 ± 0.01, 1.42 ± 0.03, t= 30.33, 18.38, 4.78, 5.75, respectively, all P < 0.05) , but significantly increased cell apoptosis rate (17.93% ± 0.88%, 17.75% ± 0.35%, 23.97% ± 0.57%, 31.44% ± 0.34%, respectively) compared the control group (3.98% ± 0.03%, 7.81% ± 0.59%, 11.95% ± 0.85%, 12.02% ± 0.48%, t= 15.93, 14.49, 11.74, 33.01, respectively, all P < 0.05) ; 48 hours after activation of LPAR6 by 2S-OMPT and ADO in Sz4 cells, compared with the control group, the LPAR6 overexpression group also showed significantly decreased cellular proliferative activity (2S-OMPT: 1.29 ± 0.04 vs. 1.48 ± 0.01; ADO: 1.27 ± 0.01 vs. 1.51 ± 0.02; both P < 0.05) , but significantly increased cell apoptosis rate (2S-OMPT: 41.70% ± 0.70% vs. 29.35% ± 0.55%; ADO: 37.05% ± 0.15% vs. 24.60% ± 1.00%; both P < 0.05) . Conclusions:LPAR6 was underexpressed in the patients with LCT, and its underexpression was significantly associated with increased risk of poor prognosis. In vitro activation of LPAR6 could inhibit the proliferation of CTCL cells and promote their apoptosis, suggesting that the decrease of LPAR6 expression may be one of the important mechanisms underlying disease progression in patients with LCT.
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Objective:To investigate clinicopathological features and prognosis of transformed mycosis fungoides (TMF) .Methods:A retrospective analysis was performed on clinicopathological data collected from 24 patients with TMF, as well as on flow cytometry results of 16 peripheral blood samples obtained from 11 of the 24 patients, who visited Hospital of Dermatology, Chinese Academy of Medical Sciences between 2014 and 2020.Results:Among the 24 patients, 11 were males and 13 were females. Their average age at diagnosis of TMF was 50.0 years (range: 18 - 77 years), and patients with early-stage TMF (9 cases) and tumor-stage TMF (15 cases) were aged 44.8 and 52.6 years on average, respectively. The average time interval from diagnosis of MF to large cell transformation was 3.7 years, and 8 patients were diagnosed with TMF at the initial visit. Histopathologically, large cells infiltrated in a diffuse pattern in 20 cases, as well as in a multifocal pattern in 4, and the proportion of large cells in 7 cases was greater than 75%. Immunohistochemically, 18 patients showed positive staining for CD30, and the proportion of CD30-positive large cells was greater than 75% in 9; negative staining for CD30 was observed in 6. Flow cytometry of 16 peripheral blood samples showed the presence of cell subsets expressing clonal T cell receptor (TCR) -vβ in 2 of 4 patients with early-stage TMF and 10 of 12 with tumor-stage TMF, and tumor cells with higher forward scatter than normal lymphocytes were detected in 16 samples. During the follow-up, among the patients with early-stage TMF, 3 progressed to tumor-stage TMF 3.3 years on average after large cell transformation, 1 progressed to erythrodermic MF in stage IIIA, and the other 4 still showed an indolent course; among the patients with tumor-stage TMF, 1 progressed to stage-IV TMF, and 5 died 3.3 (1.5 - 6) years after large cell transformation.Conclusion:Large cell transformation may occur in patients with MF in any stage, some patients have poor prognosis, so close follow-up is needed for patients with TMF.
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Objective:To explore the clinical charateristics and prognostic factors of patients with primary systemic anaplastic large cell lymphoma (ALCL).Methods:The clinicopathological data of 31 patients with newly treated primary systemic ALCL in Liaoning Cancer Hospital from January 2010 to December 2020 were retrospectively analyzed. Kaplan-Meier method was used to make survival analysis and log-rank test was performed. Multivariate analysis of factors influencing overall survival (OS) was performed by using Cox proportional hazards model.Results:Among 31 patients, there were 19 males and 12 females, with a median age of 47 years old, ranging from 16 to 74 years old. There were 18 (58.1%) patients with B symptoms, 16 (51.6%) patients with increased platelet count, 22 (71.0%) patients with Ann Arbor stage Ⅲ-Ⅳ. Until the end of follow-up, 22 cases survived and 9 cases died; the 5-year OS rate was 70.9%, and the median OS time was not reached. The 5-year OS rate of patients receiving CHOPE chemotherapy regimen was higher than that of patients receiving CHOP and other chemotherapy regimens, and the difference was statistically significant ( P = 0.049). There were statistically significant differences in the 5-year OS rates of patients with different platelet count, international prognostic index (IPI) score, chemotherapy regimens and with or without B symptoms (all P < 0.05). Multivariate analysis indicated that IPI score was an independent factor affecting OS ( HR = 2.194, 95% CI 1.078-4.465, P = 0.030). Conclusions:Most primary systemic ALCL patients are diagnosed at advanced stage and it is more common in males. Most patients have B symptoms and high platelet count. IPI score is an important prognostic factor, and CHOPE regimen may be a good choice of the first-line chemotherapy.
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Objective:To investigate the effects of autophagy-mediated crizotinib resistance on cancer stem-like cell subsets in anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALK + ALCL). Methods:The preliminary research of our group divided ALK + ALCL Karpas299 cell line into two subgroups: reporter unresponsive (RU) and reporter responsive (RR) cells through the implantation of Sox2 reporter genes, among which the RR cells had the characteristics of stem cells. Fluorescent labeled LC3 overexpressing RR and RU cells (RR-LC3 and RU-LC3) were constructed by lentiviral transfection technique, and the transfection efficiency was verified by using Western blotting and flow cytometry. RU-LC3 and RR-LC3 were treated with crizotinib at different concentrations (0, 250, 500, 1 000 nmol/L). The RED and GEN signals were detected by using double-signal flow cytometry to observe autophagy flux (RED represents the red signal B695 of the next generation of far-red fluorescent protein TagFP635 mKate; GEN represents the green signal from pH-sensitive GFP variant pHluorin B530), and the RED to GEV ratio represents autophagy flux. Real-time quantitative polymerase chain reaction (qRT-PCR) was used to detect autophagy related genes ULK1, WIPI1 and LC3B mRNA expression levels in cells. The effects of different concentrations of crizotinib (250, 500, 1 000 nmol/L) combined with chloroquine (5, 10 μmol/L) on the cell survival were detected by using MTS assay. Results:RU-LC3 and RR-LC3 cells with overexpression of LC3 were successfully constructed. After induction of 250, 500 and 1 000 nmol/L crizotinib, the RED to GEN ratio in RU-LC3 cells was 1.135±0.017, 1.453±0.017 and 1.755±0.021, respectively; the RED to GEN ratio in RR-LC3 cells was 1.193±0.018, 2.116±0.013 and 3.307±0.189, respectively; the RED to GEN ratio in RU-LC3 cells and RR-LC3 cells showed a dose-dependent manner. The RED to GEN ratio in RR-LC3 cells was higher than that in RU-LC3 cells when treated with same concentrations of crizotinib, and the differences were statistically significant (all P < 0.01). The autophagy flux of RR-LC3 cells was larger than that of RU-LC3 cells. When treated without crizotinib, mRNA relative expression levels of ULK1, WIPI1 and LC3B in RR cells were higher than those in RU cells (1.69±0.05 vs.1.01±0.02, t = -1.62, P < 0.01; 1.24±0.04 vs. 1.03±0.05, t = -2.11, P < 0.01; 1.70±0.22 vs. 1.02±0.05, t = -1.74, P = 0.033). In the absence of chloroquine, the half-inhibitory concentration ( IC50) of crizotinib in RR cells was higher than that of RU cells (950 nmol/L vs. 709 nmol/L). After treated with chloroquine, IC50 of RU cells did not change, while IC50 of RR cells was decreased with the increase of chloroquine concentration. Conclusions:Compared with RU cells, autophagy reaction of cancer stem-like RR cells is more rapid and intense, which is considered to be one of the important reasons for their resistance to crizotinib.
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Resumen En los últimos años, han aparecido evidencias que relacionan a un tipo de linfoma mamario con los implantes de silicona, lo que ha causado gran conmoción a nivel mundial. Este linfoma anaplástico de células grandes no Hodgkin (células T monoclonales), se ha visto asociado en mayoría de los casos, a las prótesis mamarias texturizadas. Es relativamente raro, ya que se puede presentar en 1 de cada 2.832 operados (as) y se puede manifestar como un seroma periprotésico o como una tumoración de la cápsula cicatrizal mamaria con o sin compromiso de la glándula y de los tejidos adyacentes.
In recent years, evidence has appeared linking a type of breast lymphoma with silicone implants, which has caused great commotion worldwide. This anaplastic large cell non-Hodgkin lymphoma (monoclonal T cells) has been associated in most cases with textured breast implants. It is relatively rare, since it can occur in 1 in 2832 operated on and it can manifest as a periprosthetic seroma or as a tumor of the mammary scar capsule with or without involvement of the gland and adjacent tissues.
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Humanos , Linfoma Anaplásico de Células Grandes , Implantes de Mama/efectos adversos , Implantación de Mama/efectos adversosRESUMEN
RESUMO A mamoplastia com próteses é uma das cirurgias plásticas mais realizadas no mundo. O processo cicatricial ao redor do implante e a presença de um biofilme pode acarretar o desenvolvimento de patologias como contratura capsular e seroma. Essas patologias parecem estar relacionadas fisiopatologicamente com o desenvolvimento do linfoma associado aos implantes mamários (BIA-ALCL), sendo este também um diagnóstico diferencial. A proposta deste trabalho é relatar o caso de uma paciente com desconforto mamário, que após 2 cirurgias prévias para drenagem de seroma e troca de próteses, apresentava desconforto mamário e alteração em exames de imagens da mama esquerda. Sendo submetida a uma capsulectomia em bloco da mama esquerda e completa à direita, tendo suas próteses substituídas. Os exames para investigação de BIA-ALCL foram negativos e os achados patológicos foram sugestivos de contratura capsular à esquerda e formação de dupla cápsula à direita. O trabalho enfatiza a importância do diagnóstico diferencial em patologias mamárias, o acompanhamento a longo prazo e medidas de profilaxia na formação do biofilme.
Abstract Mammoplasty with prostheses is one of the most performed plastic surgeries in the world. However, the healing process around the implant and the presence of a biofilm can lead to the development of pathologies such as capsular contracture and seroma. These pathologies seem to be physiologically related to the development of lymphoma associated with breast implants (BIA-ALCL), which is also a differential diagnosis. The purpose of this study is to report the case of a patient with breast discomfort who, after two previous surgeries for seroma drainage and prosthesis replacement, presented breast discomfort and alteration in imaging of the left breast. She was, submitted to a capsulectomy in a block of the left breast and complete on the right, having her prostheses replaced. Bia-ALCL investigation tests were negative and pathological findings were suggestive of left capsular contracture and double capsule formation on the right. The study emphasizes the importance of differential diagnosis in mammary pathologies, long-term follow-up, and prophylaxis measures in biofilm formation.
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OBJECTIVE@#To explore the strategy of pregnancy-preserving and maternal- fetal management in patients with primary gynecologic neuroendocrine tumors (gNETs) during pregnancy.@*METHODS@#We performed whole genome sequencing (WGS) for analyzing maternal and fetal somatic and germline single nucleotide variations (SNVs) and small insertions and deletions (InDels) for a 29-year-old pregnant woman diagnosed with stage IB2 large cell neuroendocrine carcinoma (LCNEC) and adenocarcinoma in the cervix. A systematic literature review was performed to explore the strategies for treatment of such rare histological type while maintaining pregnancy.@*RESULTS@#Global case analysis of cervical NETs during pregnancy suggested that negative lymph node metastasis and an early FIGO stage were potentially associated with a good prognosis of the patients. In the case presented herein, a pregnancy-preserving strategy was adopted and favorable maternal-fetal outcomes were achieved after neoadjuvant chemotherapy, radical surgery and postoperative systemic chemotherapy. At 35@*CONCLUSIONS@#Although gNETs in pregnancy are rare and highly risky, pregnancy-preserving managements of gNETs can still be considered and favorable maternalfetal outcomes are possible with proper assessment of the clinical indications and implementation of multimodal treatments. Precise treatment and follow-up strategies based on the results of WGS for risk-reducing intervention of cancer recurrence or occurrence can potentially benefit the patient and the neonate.
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Adulto , Niño , Femenino , Humanos , Recién Nacido , Embarazo , Adenocarcinoma , Carcinoma Neuroendocrino/genética , Recurrencia Local de Neoplasia , Neoplasias del Cuello Uterino/genéticaRESUMEN
BACKGROUND@#Studies have shown that elevated serum lactate dehydrogenase (LDH) concentration can lead to poor prognosis in patients with small cell lung cancer and lung adenocarcinoma, but its relationship with the prognosis of patients with lung large-cell neuroendocrine carcinoma (L-LCNEC) is not clear. This study aims to explore the influence of L-LCNEC preoperative serum LDH concentration and postoperative LDH concentration change trend on the disease-free survival (DFS) of patients after surgery, so as to judge the clinical prognosis of L-LCNEC provides new ideas.@*METHODS@#Collected the clinical data. The receiver operating characteristic (ROC) curve was used to determine the optimal cut-off value, while the Kaplan-Meier and Cox proportional hazard model were used to analyze data.@*RESULTS@#DFS was shortened in patients with high serum LDH concentration before operation and increased LDH concentration after operation (P<0.001, P<0.001). The preoperative LDH concentration and postoperative LDH concentration change trend were independent prognostic factors for patients (P<0.001, P=0.037).@*CONCLUSIONS@#Preoperative LDH concentration and its postoperative concentration change trend in patients with L-LCNEC are independent prognostic factors for DFS of patients.
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Anaplastic lymphoma kinase (ALK) positive, anaplastic large cell lymphoma involving the non-mammary implant is an extremely rare presentation. Irrespective of the type or site, the implant-associated primary ALCL is morphologically and immunophenotypically similar to ALK-negative ALCLs. Herein, we present the case of a 42-year-old male who developed a lytic lesion after an implant for a right femur fracture. The lytic lesion biopsy revealed anaplastic large cell lymphoma with ALK protein expression. Imaging findings showed the widespread dissemination of disease all over the body, entrapping the implant too. ALCL involving the bone implant is a very unusual and rare presentation that needs to be documented.
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Humanos , Masculino , Adulto , Linfoma Anaplásico de Células Grandes , Fracturas del Fémur/complicaciones , Quinasa de Linfoma Anaplásico , Prótesis e ImplantesRESUMEN
Cardiac lymphoma is a rare entity. In this setting, the secondary involvement of the heart is far more frequent than the primary cardiac lymphoma. Herein, we present an autopsy case of a disseminated anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma with a dominant mediastinal involvement. Extensive cardiac infiltration with the near replacement of the myocardial wall by the neoplastic cells was observed. A total of nine isolated case reports of anaplastic large cell lymphoma with cardiac involvement were found in the English-language literature, and a widespread cardiac and thymic infiltration by the systemic ALK-positive anaplastic large cell lymphoma has not been documented. An incidental regenerative nodule was also identified in the liver. The patient died of pulmonary thromboembolism and cardiac arrest.
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Humanos , Femenino , Adulto , Linfoma Anaplásico de Células Grandes/patología , Neoplasias Cardíacas , Autopsia , Tromboembolia , Timo/patología , Resultado Fatal , Quinasa de Linfoma Anaplásico , Paro CardíacoRESUMEN
Objective:To investigate the clinical features and prognosis of primary central nervous system (CNS) anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) in children.Methods:The clinical data of a child with primary CNS ALK-positive ALCL in Fujian Medical University Union Hospital were retrospectively analyzed, and the relevant literature was reviewed.Results:The child went to other hospitals with headache and fever as the main symptoms. Head magnetic resonance imaging showed a right cerebellar mass, and there was no evidence of lymphoma infiltration outside the CNS before surgery. Later, cerebellar tumor resection was performed. After the surgery, through pathological examination, the child was diagnosed as ALK-positive ALCL, but did not receive chemotherapy in time. The child transferred to Fujian Medical University Union Hospital on the 27th day after surgery, and the tumor had spread to bone marrow, testis, vertebrae, etc., and the peripheral blood NPM-ALK fusion gene was positive. The child received 2 courses of chemotherapy and achieved complete remission, but eventually died of chemotherapy complications.Conclusions:Primary CNS ALK-positive ALCL is rare and easy to be misdiagnosed. The disease progresses quickly, and the overall prognosis is poor. Timely biopsy for diagnosis and early comprehensive treatment based on chemotherapy may improve the prognosis of patients.
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A 43 year old female presented to the emergency department for nausea, vomiting and abdominal pain secondary to a bowel obstruction subsequently revealed to be a poorly differentiated large cell neuroendocrine tumor of the colon. After a CT scan showed a mass in the ascending colon with possible metastasis to the right lobe of the liver, an exploratory laparotomy was performed. A hemicolectomy was performed with biopsy of the liver mass. Pathology was consistent with large cell neuroendocrine tumor in all specimens including the liver biopsy, ascending colon, and transverse colon. Although large cell neuroendocrine tumors of the colon are a rare malignancy, they are an important consideration in the workup of multiple colonic masses with metastases, especially in patients presenting with bowel obstruction. The literature on poorly differentiated large cell neuroendocrine cancer and treatment is reviewed. Poorly differentiated large cell neuroendocrine tumor is a rare pathology but should be included in the differential diagnosis in patients presenting with a colon mass and bowel obstruction.